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Updated: Jan 8, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Systemic Sclerosis-Associated Interstitial Lung Disease: What We Know and How to Incorporate Guidelines Into Clinical

Anthony J Esposito¹, Kavitha C Selvan¹, Carrie Richardson²

¹Division of Pulmonary and Critical Care, Chicago, IL.

|December 15, 2025

Summary

Systemic sclerosis-interstitial lung disease (SSc-ILD) management is complex. This review synthesizes guidelines for screening and treatment, emphasizing mycophenolate as the strongly recommended therapy for SSc-ILD.

Keywords:

guidelines interstitial lung disease screening systemic sclerosis treatment

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Published on: March 6, 2019

Area of Science:

Rheumatology
Pulmonology
Clinical Practice Guidelines

Background:

Interstitial lung disease (ILD) affects a significant portion of systemic sclerosis (SSc) patients, being a leading cause of mortality.
Limited treatment options and contrasting clinical practice guidelines complicate SSc-ILD management.
Recent guidelines offer diverse perspectives on treating SSc-ILD, posing challenges for clinicians.

Purpose of the Study:

To synthesize current evidence on SSc-ILD management.
To guide the practical application of recent clinical practice guidelines for SSc-ILD.
To provide a framework for screening and treatment decision-making in SSc-ILD.

Main Methods:

Review of randomized controlled trials (RCTs) and clinical practice guidelines.
Synthesis of evidence on screening protocols for SSc-ILD.
Appraisal of therapeutic options for SSc-ILD based on guideline recommendations.

Main Results:

High-resolution computed tomography (HRCT) is recommended for initial SSc-ILD screening, with re-screening advised for high-risk patients or those with new symptoms.
Mycophenolate is the only therapy strongly recommended for SSc-ILD, despite low evidence quality for most treatments.
Guidelines conditionally recommend mycophenolate, tocilizumab, rituximab, cyclophosphamide, and nintedanib.

Conclusions:

Current SSc-ILD diagnosis and treatment approaches are appraised.
Gaps in knowledge are identified, paving the way for future research.
A precision-guided approach to SSc-ILD diagnosis and treatment is proposed.